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63 Cards in this Set

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  • Back
  • 3rd side (hint)
Intial pathology in Alzheimer's is where in the brain?
hippocampus and entorhinal cortex
Features of Lewy Body Dz
mild parkinsons, early dementia
REM sleep d/o
what are lewy bodies made of?
alpha synuclein (intranuclear inclusions)
What are the 3 types of frontotemporal lobar degeneration?
Frontal Temporal Dementia
Primary Progressive Aphasia
Semantic Dementia
Characteristics/location of frontal temporal dementia
Mostly frontal lobes
personality changes, impaired social conduct, disinhibited and perseverative behavior
Characteristics/location of primary progressive aphasia?
left frontal lobe
non-fluent speech but preserved understanding (Broca's aphasia)
Characteristics/location of semantic dementia?
temporal lobes
loss of understanding...fluent, meaningless speech (wernicke's aphasia)
What are Pick bodies?
inclusion bodies which stain for tau protein found in frontal temporal lobe degeneration dzs.
Tx for ALS?
Riluzole and supportive
UMN vs LMN signs
UMN - hyper reflexia
LMN - fasiculations and atrophy
How does ALS present?
UMN and LMN signs
normal sensory and intellect
usually asymmetric
Genetics of Spinal muscular atrophy?
AR (Survival motor neuron on 5q)
Werdnig Hoffman Syndrome signs/sxs?
LMN sxs
floppy baby
tongue fasiculations
Kennedy Dz genetics and sxs? lifespan?
spinobulbar muscular atrophy
X-linked CAG repeat androgen receptor defect
LMN w/gyecomastia
lifespan is normal
Dx of Guillain-Barre syndrome? presentation?
increased protein in CSF
sxs: ascending weakness, arreflexia
What disease is:
distal greater than proximal weakness, foot drop, atrophy and numbness
Charcot-Marie-Tooth dz
most common is uniform demyelination
What is multiple mononeuropathy suggestive of?
nerve infarction/inflammation
vasculitic neuropathies (lupus/polyarteritis nodosa)
How do you diagnose a small fiber neuropathy?
biopsy (not EMG or NCS)
Tx for Guillain Barre?
no roids
give IVIg and plasma exchange
Dx for Chronic Inflammatory Demyelinating Polyradiculoneuropathy?
EMG shows conduction block and temporal dispersion
Name the disease:
acute onset of arm pain associated with patchy weakness and numbness
cannot form "OK"
Parsonage-Turner Syndrome
What other conditions is Parsonage-Turner associated with?
DM, SLE, vasculitis
Which neuromuscular junction dz is presynaptic? postsynaptic?
pre-synpatic - Lambert Eaton, Botulism
post-synaptic - Myasthenia Gravis
What tumor is associated with myasthenia gravis?
inheritance pattern of congenital myasthenic syndromes?
Histologic difference btw LEMS and MG
LEMS - increased post-synaptic folds
MG - decreased post-synaptic folds
Which disorders have proximal weakness more than distal weakness?
NM junction disorders
What are typical findings in myopathies?
elevated CK or CPK
proximal weakness
usefull tests and results for myopathies?
EMG - short duration/small amplitude APs
CK/aldolase - elevated
Bx - metabolic deposits
Pathophys of muscular dystrophies?
reduced or absent expression of protein product of dsytrophin (X-linked)
Features of Duchenne musc dyst?
calf pseudohypertrophy
gowers sign
mental retardation
How is Becker's musc dyst different from Duchenne?
less MR
less cardiomyopathy
later onset
normal lifespan
Histopath of IBM? Tx?
beta-amyloid deposits (alzheimer's of the muscle)
no tx
Diagnostic tests for toxic myopathies?
check CK
(often a normal EMG)
3 examples of mitochondiral myopathies
MERRF - myoclonic epilepsy, ragged red fibers
MELAS - mitoch encephalomyopathy lactic acidosis stroke
MNGIE - mitoch neurogastrointestinal encephalopathy syndrome
Family history of DM, short stature, sz, deafness indicates what disease?
mitochondrial myopathies
What are ragged red fibers?
accumulations of abnormal mitochondria
How do mitochondrial myopathy pts present?
ptosis, ophthalmoplegia
When do you get periodic paralyses?
after large carbohydrate meals and rigorous exercise, stress
HYPOkalemic paralysis
HYPERkalemic paralysis
HYPO - Ca channel, hrs to days tx with acetazolamide
HYPER - Na channel, mins to hrs
Which brain tumors are ass w/ NF1?
pilocytic astrocytomas of the optic pathways and cerebellum
which brain tumors are ass w/ NF2?
bilateral acoustic schwannomas
Where do brain mets come from?
lung > breast > melanoma > colon
Treatment of Choice for multiple brain mets?
whole brain radiation
Treatment for single brain met?
resection (gamma if not resectable) followed by whole brain rad
Imaging modality for brain tumors?
MRI w/ and w/o contrast
Treatment for low grade gliomas?
Maximum Surgical resection
Treatment for high grade glioma?
Resection + RT + (temozolomide)
What predicts better survival in pts with high grade gliomas?
younger age, methylated MGMT gene
Treatment for medulloblastoma?
resection + craniospinal RT + chemo
What type of tumor is a medulloblastoma and what are the symptoms?
primitive neurectodermal tumor. HA, N/V, ataxia
What are rosenthal fibers associated with?
pilocytic astrocytomas (eosinophilic corkscrew fibers), alexander dz?
How is a cluster HA different from a migraine?
clusters are shorter, regular patterns, faster onset, piercing pain
Episodic vs. Chronic tension or migraine HA?
chronic is on more than 15 days/month
You must have what sxs during a HA to classify as a migraine?
either N/V or photophobiaANDphonophobia
When is the migraine prodrome? Aura?
prodrome - hours before HA…aura - minutes before
What is the most important risk factor for cluster HA?
What is the occurrence pattern in cluster HA?
once or twice a day for 6-8wks. Once or twice/year
What is the defect in FHM1?
Ca channel disorder that leads to elevated Glu in the cortex
What causes the pain in migraine?
sterile trigeminal nerve inflammation
Where is the generator/modulator in migraines?
rostral pons and midbrain tegmentum
What is allodynia in migraine due to?
central sensitization
Red flags for HA?
Systemic,Secondary; Neurologic sxs; suddOnset; Older; Previous hx